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[与透析相关的淀粉样变性]

[Amyloidosis associated with dialysis].

作者信息

Mátyus J, Kakuk G

机构信息

Debreceni Orvostudományi Egyetem, I. Belklinika.

出版信息

Orv Hetil. 1995 Mar 12;136(11):587-93.

PMID:7700615
Abstract

Recently dialysis related amyloidosis has become a major complication in patients treated with long-term dialysis therapy. The serum level of the amyloid precursor beta 2-microglobulin is significantly elevated in uraemia, mostly due to the retention. The bioincompatibility of dialysis membranes and the endotoxin content of the dialysate may contribute to the synthesis and tissue deposition of beta 2-microglobulin, but the details of pathogenesis are not yet cleared. At first periarticular and perineural structures are involved in the deposition of amyloid. The carpal tunnel syndrome is of great differential diagnostic value, it appears frequently together with the beginning of the joint pain. The main target of arthropathy are the large and medium-sized joints symmetrically. Deposition of the amyloid to the subchondral bone cysts might lead to pathological fractures, mainly in the hips and destructive spondylarthrophathy might involve severe neurologic complications. Visceral organs (gastrointestinal and urogeniteal tract, heart etc.) are involved rarely and later. Ultrasonography and isotope methods in addition to the conventional radiologic examinations are also used to differentiate the joint complaints nowadays. The definitive diagnosis is based on immunohistology. The alteration of dialysis strategy first of all the usage of high permeable, biocompatibile membranes and pure dialysis water has a role in the prevention of disease and decreasing its progression. In the case of developed lesions timely surgical-orthopedic interventions are required in addition to drug therapy. Todays' renal transplantation is a successful treatment, but the consequences of amyloid depositions already formed can't be left out of considerations even after transplantation.

摘要

近年来,透析相关淀粉样变已成为长期接受透析治疗患者的主要并发症。尿毒症患者血清中淀粉样前体β2-微球蛋白水平显著升高,主要是由于潴留所致。透析膜的生物不相容性和透析液中的内毒素含量可能会促进β2-微球蛋白的合成和组织沉积,但发病机制的细节尚不清楚。起初,淀粉样物质沉积于关节周围和神经周围结构。腕管综合征具有重要的鉴别诊断价值,常与关节疼痛同时出现。关节病的主要累及部位是对称的大中型关节。淀粉样物质沉积于软骨下骨囊肿可能导致病理性骨折,主要发生在髋部,而破坏性脊椎关节病可能会引发严重的神经并发症。内脏器官(胃肠道、泌尿生殖系统、心脏等)很少且较晚受累。除了传统的放射学检查外,超声检查和同位素方法如今也被用于鉴别关节疾病。确诊依靠免疫组织学检查。改变透析策略,首先是使用高通透性、生物相容性好的膜和纯透析用水,对预防疾病和减缓其进展具有作用。对于已形成病变的情况,除药物治疗外,还需要及时进行外科骨科干预。如今肾移植是一种成功的治疗方法,但即使在移植后,也不能忽视已形成的淀粉样物质沉积的后果。

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Orv Hetil. 1995 Mar 12;136(11):587-93.
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